Primary and Secondary Stroke Prevention in Children With Sickle Cell Disease

J Pediatr Health Care. 2017 Mar-Apr;31(2):145-154. doi: 10.1016/j.pedhc.2016.06.005. Epub 2016 Jul 14.

Abstract

Children with sickle cell disease (SCD) have numerous acute and chronic complications, including central nervous system (CNS) disease, which can be debilitating over their life span. Recognition of risk factors for CNS disease and overt CNS disease should be properly identified by primary care providers, including physicians, physician assistants, and nurse practitioners. Here, we discuss an emerging and important early indicator of CNS disease in the form of silent cerebral infarcts and review overt stroke in patients with SCD. We also discuss transcranial Doppler ultrasonography, when and how often transcranial Doppler ultrasounds should be performed, and management of abnormal results. Lastly, we review the clinical data for the management and prevention of silent cerebral infarcts and overt stroke in children with SCD.

Keywords: Cerebral infarct; sickle cell disease; stroke; transcranial Doppler ultrasonography.

MeSH terms

  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / diagnostic imaging
  • Anemia, Sickle Cell / physiopathology*
  • Antisickling Agents / pharmacology
  • Antisickling Agents / therapeutic use*
  • Blood Transfusion
  • Cerebrovascular Circulation
  • Child
  • Computed Tomography Angiography
  • Humans
  • Hydroxyurea / pharmacology
  • Hydroxyurea / therapeutic use*
  • Magnetic Resonance Angiography
  • Practice Guidelines as Topic
  • Practice Patterns, Physicians'
  • Primary Prevention / methods*
  • Secondary Prevention / methods*
  • Stroke / etiology*
  • Stroke / physiopathology
  • Stroke / prevention & control*
  • Ultrasonography, Doppler, Transcranial / statistics & numerical data

Substances

  • Antisickling Agents
  • Hydroxyurea